慢性家族性良性天疱疮误诊为慢性湿疹1例

A Case of Chronic Familial Benign Pemphigus Misdiagnosed as Chronic Eczema

患者女,62岁。躯干、四肢皮肤皱褶处红斑、丘疹、斑块伴瘙痒20余年。先后在多家医院均按慢性湿疹治疗,无明显疗效。皮损组织病理示:表皮灶状角化不全,棘层增生肥厚,可见灶状角化不良细胞和轻度的棘刺松解现象。真皮血管周围灶状炎症细胞浸润。诊断:慢性家族性良性天疱疮。

A 62-year-old female presented with erythema, papules and plaques and rugae on her trunk and limbs with itching twenty years before. She had been clinically treated as chronic eczema in different hospitals, without obvious improvement histopathological examination：focal parakeratosis acanthosis. There were focal dyskera- totic cell and mild released of thorns in the epidermis. Perivascular infiltration of focal inflammatory cells in dermis was observed. This patient was diagnosed as familial benign chronic pemphigus.